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Epidermolysis Bullosa Acquisita

By Daniel M. Peraza, MD, The Geisel School of Medicine at Dartmouth

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Epidermolysis bullosa acquisita is a rare, acquired, chronic condition characterized by subepidermal blistering.

Epidermolysis bullosa acquisita can occur in all ages. Collagen type VII, the major component of the anchoring fibrils, is the target antigen of this autoimmune disorder. Multiple myeloma, amyloidosis, lymphoma, inflammatory bowel disease, and systemic lupus erythematosus increase the risk of having epidermolysis bullosa acquisita.

Symptoms and Signs

Initial manifestations are highly variable, sometimes resembling those of bullous pemphigoid. Bullous lesions are most often in areas subject to minor trauma, such as the extensor aspects of the elbows and the dorsal aspects of the hands and feet. Healing usually causes scars, milia (superficial epidermal inclusion cysts), and hyperpigmentation. Some patients have dystrophic nails, mucosal involvement, or ocular lesions leading to blindness.

Diagnosis

  • Skin biopsy and direct immunofluorescence

Diagnosis of epidermolysis bullosa acquisita is confirmed by skin biopsy and direct immunofluorescence.

Salt-split skin (skin incubated with sodium chloride to separate the sample into zones) for indirect immunofluorescence may be needed for differentiation from bullous pemphigoid.

Treatment

  • Corticosteroids and dapsone

The prognosis is variable, but disease course tends to be prolonged. High-quality evidence about treatments is lacking, and treatment recommendations are often anecdotal. However, in children, corticosteroids in combination with dapsone have shown benefit. In adults and in people with more severe disease, corticosteroids, dapsone, colchicine, cyclosporine, mycophenolate mofetil, IV immune globulin, and azathioprine have been reported to be successful.

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